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Isolated cystic dilatation of the cystic duct
CASE REPORTS
Isolated Cystic Dilatation of the Cystic Duct
William E. Bode, MD, San Antonio, Texas J. Bradley Aust, MD, PhD, San Antonio, Texas
Congenital cystic dilatation of the extrahepatic biliary tree is a relatively unusual condition. Traditionally, it has been classified into three types [I]. Type I is a fusiform dilatation of the common bile duct, which may or may not include the common hepatic duct; type II is a diverticulum from the side of the common bile duct; and type III is a choledochocele or cystic dilatation of the intraduodenal portion of the common bile duct. We present an unusual and previously unreported variation of congenital cystic dilatation of the extrahepatic biliary tree and its management. Case Report A 7 year old Mexican-American girl presented to the pediatric emergency room with a 3.5 month history of painless, asymptomatic jaundice. She had previously enjoyed good health, and a review of her old records failed to show prior evidence of jaundice or hyperbilirubinemia. Her total bilirubin value was 5.5 mg/lOO ml, alkaline phosphatase 825 IU/liter, serum glutamic oxalecetic transaminase (SGOT) 129 II-l/liter, and serum glutamic pyruvic transaminase (SGPT) 162 Ill/liter. Even though hepatitis-associated antigen was negative, she was diagnosed as having hepatitis and was sent home with a follow-up appointment. She did not return for the scheduled follow-up until 2 weeks before admission, at which time she presented again to the pediatric emergency room with a total bilirubin value of 5.9 mg/lOO ml, alkaline phosphatase 2,200 IU/liter, SGOT 236 II-l/liter, and SGPT 300 IU/liter. Her examination remained unremarkable, except for the jaundice, mild arthralgia, and pruritis. Once again the diagnosis was hepatitis and she was sent home with a 2-week follow-up appointment. On her return it was noted that she continued to have pruritis, arthralgia, and deepening jaundice. She was afebrile and her abdominal examination revealed no abnormalities. The total bilirubin value was 14.1 mg/lOO ml, alkaline phosphatase 1,640 III&r, SGOT 261 IU/liter, SGPT 263 IU/liter, and the prothrombin time was 4 s out. Her complete blood count, electrolytes, urinalysis, chest From the Department of Surgery. The University of Texas Health Science Center at San Antonio, San Antonio, Texas. Requests for reprints should be addressed to J. Bradley Aust, MD, Department of Surgery, The University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, Texas 78284.
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roentgenogmm, and abdominal flat plate did not reveal any abnormalities. She was admitted to the pediatric service with a diagnosis of obstructive jaundice. A sonogram showed multiple cystic areas in both the right and left lobes of the liver, a normal gallbladder, and no common bile duct dilatation. These findings were reported as being consistent with Caroli’s disease. Technetium-99m-para-isopropylacetanilidoiminodiacetic acid. scan suggested hepatocellular disease and showed intrahepatic biliary tract dilatation with a patent common bile duct and visualization of the gallbladder. Endoscopic retrograde cholangiopancreatography was attempted, but failed because of poor patient cooperation. Consultation with the surgical service revealed a normally developed, jaundiced child, with an otherwise unremarkable examination. Her total bilirubin value had decreased to 5 mg/lOO ml and her other laboratory values were unchanged. She start receiving gentamicin, clindamycin, and ampicillin, and an immediate percutaneous transhepatic cholangiogram was obtained. This showed complete obstruction of the common bile duct at the margin of the duodenum, diffuse noncystic dilatation of the intrahepatic ducts, and what was interpreted as a probable type II choledochal cyst. The gallbladder and cystic duct were not visualized. She was given vitamin K and fresh frozen plasma, and was taken to surgery the next morning with a diagnosis of choledochal cyst and common bile duct obstruction. At exploration, an inflamed 6 cm cyst confined to the cystic duct was found. The cystic duct entered the common bile duct about 1 cm proximal to the duodenal margin. The common bile duct was obstructed secondary to inflammation and mass effect at the duodenal margin, and was enlarged secondary to obstruction but was not cystic. Examination of the cyst and common bile duct exploration failed to reveal any stones (Figure 1). The gallbladder was taken down, the cyst was dissected free, and the cystic duct was transected at its junction with the common bile duct. A duodenostomy was performed and careful intraduodenal examination ruled out a choledochocele. A choledochoduodenostomy was performed. The patient did well postoperatively and went home in 1 week. At follow-up 3 weeks later, her total bilirubin value was 0.8 mg/lOO ml, alkaline phosphatase 419 IU/liter, SGOT 76 IU/liter, SGPT 82 IU/liter, and her prothrombin time was within normal limits.
The American Journal of Surgery
Dilatation
of the Cystic Duct
Comments The patient described had a unique presentation of congenital cystic dilatation of the extrahepatic biliary tract, one not covered by the standard classification of Alonso-Lej et al [I]. The principles of management of congenital cystic dilatation of the extrahepatic biliary tract should include (1) prompt diagnosis; (2) careful examination of the entire biliary tract; (3) total excision of the cyst (there is a 2 to 3 percent incidence of cyst carcinoma associated with leaving these cysts in place [2,3]); (4) reestablishment of biliary tract and intestinal continuity; and (5) cholecystectomy to prevent formation of stones or acalculus cholecystitis in the future
[3,41. Summary Presented was an unusual case of congenital cystic dilatation of the extrahepatic biliary tract in which the cystic changes were confined solely to the cystic duct. There was concomitant common bile duct obstruction secondary to inflammation and mass effect from the cyst. Management consisted of cholecystectomy, excision of the cyst, common bile duct exploration, examination of the ampullary region by duodenotomy, and choledochoduodenostomy. References 1. Alonsdej F, Rever WB, Pessango DJ. Congenital choledochal cyst, with a report of 2, and an analysis of 94 cases. Int Abstr Surg 1959; 108: 1. 2. Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433
Volume 145, June 1953
Figure 1. Anatomic arrangement encountered at time of expioration: 1, cyst of the cyetk duct and 2, area of common bile duct obetruction.
patients in the Japanese literature. Am J Surg 1980;140: 653-7. 3. Oldham KT, Hart MJ, White TT. Choledochal cysts presenting in late childhood and adulthood. Am J Surg 1981;141:56871. 4. Flanigan PD. Biliary cysts. Ann Surg 1975;182:635-43.
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Isolated Cystic Dilatation of the Cystic Duct
William E. Bode, MD, San Antonio, Texas J. Bradley Aust, MD, PhD, San Antonio, Texas
Congenital cystic dilatation of the extrahepatic biliary tree is a relatively unusual condition. Traditionally, it has been classified into three types [I]. Type I is a fusiform dilatation of the common bile duct, which may or may not include the common hepatic duct; type II is a diverticulum from the side of the common bile duct; and type III is a choledochocele or cystic dilatation of the intraduodenal portion of the common bile duct. We present an unusual and previously unreported variation of congenital cystic dilatation of the extrahepatic biliary tree and its management. Case Report A 7 year old Mexican-American girl presented to the pediatric emergency room with a 3.5 month history of painless, asymptomatic jaundice. She had previously enjoyed good health, and a review of her old records failed to show prior evidence of jaundice or hyperbilirubinemia. Her total bilirubin value was 5.5 mg/lOO ml, alkaline phosphatase 825 IU/liter, serum glutamic oxalecetic transaminase (SGOT) 129 II-l/liter, and serum glutamic pyruvic transaminase (SGPT) 162 Ill/liter. Even though hepatitis-associated antigen was negative, she was diagnosed as having hepatitis and was sent home with a follow-up appointment. She did not return for the scheduled follow-up until 2 weeks before admission, at which time she presented again to the pediatric emergency room with a total bilirubin value of 5.9 mg/lOO ml, alkaline phosphatase 2,200 IU/liter, SGOT 236 II-l/liter, and SGPT 300 IU/liter. Her examination remained unremarkable, except for the jaundice, mild arthralgia, and pruritis. Once again the diagnosis was hepatitis and she was sent home with a 2-week follow-up appointment. On her return it was noted that she continued to have pruritis, arthralgia, and deepening jaundice. She was afebrile and her abdominal examination revealed no abnormalities. The total bilirubin value was 14.1 mg/lOO ml, alkaline phosphatase 1,640 III&r, SGOT 261 IU/liter, SGPT 263 IU/liter, and the prothrombin time was 4 s out. Her complete blood count, electrolytes, urinalysis, chest From the Department of Surgery. The University of Texas Health Science Center at San Antonio, San Antonio, Texas. Requests for reprints should be addressed to J. Bradley Aust, MD, Department of Surgery, The University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, Texas 78284.
828
roentgenogmm, and abdominal flat plate did not reveal any abnormalities. She was admitted to the pediatric service with a diagnosis of obstructive jaundice. A sonogram showed multiple cystic areas in both the right and left lobes of the liver, a normal gallbladder, and no common bile duct dilatation. These findings were reported as being consistent with Caroli’s disease. Technetium-99m-para-isopropylacetanilidoiminodiacetic acid. scan suggested hepatocellular disease and showed intrahepatic biliary tract dilatation with a patent common bile duct and visualization of the gallbladder. Endoscopic retrograde cholangiopancreatography was attempted, but failed because of poor patient cooperation. Consultation with the surgical service revealed a normally developed, jaundiced child, with an otherwise unremarkable examination. Her total bilirubin value had decreased to 5 mg/lOO ml and her other laboratory values were unchanged. She start receiving gentamicin, clindamycin, and ampicillin, and an immediate percutaneous transhepatic cholangiogram was obtained. This showed complete obstruction of the common bile duct at the margin of the duodenum, diffuse noncystic dilatation of the intrahepatic ducts, and what was interpreted as a probable type II choledochal cyst. The gallbladder and cystic duct were not visualized. She was given vitamin K and fresh frozen plasma, and was taken to surgery the next morning with a diagnosis of choledochal cyst and common bile duct obstruction. At exploration, an inflamed 6 cm cyst confined to the cystic duct was found. The cystic duct entered the common bile duct about 1 cm proximal to the duodenal margin. The common bile duct was obstructed secondary to inflammation and mass effect at the duodenal margin, and was enlarged secondary to obstruction but was not cystic. Examination of the cyst and common bile duct exploration failed to reveal any stones (Figure 1). The gallbladder was taken down, the cyst was dissected free, and the cystic duct was transected at its junction with the common bile duct. A duodenostomy was performed and careful intraduodenal examination ruled out a choledochocele. A choledochoduodenostomy was performed. The patient did well postoperatively and went home in 1 week. At follow-up 3 weeks later, her total bilirubin value was 0.8 mg/lOO ml, alkaline phosphatase 419 IU/liter, SGOT 76 IU/liter, SGPT 82 IU/liter, and her prothrombin time was within normal limits.
The American Journal of Surgery
Dilatation
of the Cystic Duct
Comments The patient described had a unique presentation of congenital cystic dilatation of the extrahepatic biliary tract, one not covered by the standard classification of Alonso-Lej et al [I]. The principles of management of congenital cystic dilatation of the extrahepatic biliary tract should include (1) prompt diagnosis; (2) careful examination of the entire biliary tract; (3) total excision of the cyst (there is a 2 to 3 percent incidence of cyst carcinoma associated with leaving these cysts in place [2,3]); (4) reestablishment of biliary tract and intestinal continuity; and (5) cholecystectomy to prevent formation of stones or acalculus cholecystitis in the future
[3,41. Summary Presented was an unusual case of congenital cystic dilatation of the extrahepatic biliary tract in which the cystic changes were confined solely to the cystic duct. There was concomitant common bile duct obstruction secondary to inflammation and mass effect from the cyst. Management consisted of cholecystectomy, excision of the cyst, common bile duct exploration, examination of the ampullary region by duodenotomy, and choledochoduodenostomy. References 1. Alonsdej F, Rever WB, Pessango DJ. Congenital choledochal cyst, with a report of 2, and an analysis of 94 cases. Int Abstr Surg 1959; 108: 1. 2. Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433
Volume 145, June 1953
Figure 1. Anatomic arrangement encountered at time of expioration: 1, cyst of the cyetk duct and 2, area of common bile duct obetruction.
patients in the Japanese literature. Am J Surg 1980;140: 653-7. 3. Oldham KT, Hart MJ, White TT. Choledochal cysts presenting in late childhood and adulthood. Am J Surg 1981;141:56871. 4. Flanigan PD. Biliary cysts. Ann Surg 1975;182:635-43.
829